Sickle Cell Anemia Pathophysiology, Podcast, and Nursing Care Plan

Sickle Cell Anemia

Sickle cell anemia is a form of anemia that is inherited. In this condition there is not an adequate number of healthy red blood cells to transport enough oxygen to your organs and tissues.

Usually, red blood cells are concave in appearance. Because of their shape, the cells are flexible and can move easily through your body via blood vessels.

Sickle cell anemia occurs when the red blood cells become shaped like sickles or crescent moons. When this happens they become sticky and rigid causing the red blood cells to become stuck in small blood vessels. As this happens, blood flow is blocked or slowed depriving the body of the oxygen that it needs. Currently, there is no cure for sickle cell anemia.

This gene is more common in families from Africa, Saudi Arabia, Caribbean islands, Mediterranean, and South and Central America. In the United States, sickle cell anemia most commonly effects African American families.

Causes

A genetic mutation is the cause of sickle cell anemia. The hemoglobin caused by sickle cell anemia is sticky, has a sickle shape, and is rigid. The sickle gene is passed genetically from generation to generation. Both parents must be a carrier of the defective gene to pass it to their child. This is known as autosomal recessive inheritance. However, if only one parent has the gene, the child will have the sickle cell trait. Patients with the sickle cell anemia trait make both normal hemoglobin and sickle cell hemoglobin. Although the blood may contain some sickle cells, patients usually do not experience any symptoms. But, they are still a carrier of the disease, so they can still pass the defective gene to their babies.

If two people with the sickle cell anemia trait have a child, they have:

a 25% chance of having a child without the trait and with normal hemoglobin.

a 50% chance of have a child whom is a carrier of the sickle cell anemia trait.

a 25% chance of having a child with sickle cell anemia.

Signs and Symptoms

Signs of sickle cell anemia manifest as early as 4 months of age, which may include:

Anemia – Sickle cells are fragile and break apart easily. Normal red blood cells live for approximately 120 days. Sickle cells only live for about 20 days. This shortage of red blood cells (RBC’s) causes anemia. Without an adequate amount of RBC’s, the body will not be able to get enough oxygen. This can result in shortness of breath, dizziness, headaches, cold extremities, and paler.

Pain – Sickle cell crises is when the patient experiences periodic episodes of pain. The sickle shaped RBC’s impede blood flow through small blood vessels in abdomen, chest and joints causing pain. The pain can last hours or weeks and vary in intensity. If pain is severe enough, the patient will need to be hospitalized.

Hand-foot syndrome – Swollen feet and hands may be the first sign in babies.

Infection – The spleen which is an organ that fights infection may become damaged by sickled cells.

Growth delays – Sickled cells rob the patients body of the much needs oxygen and nutrients necessary for growth.

Visual challenges – The blood vessels that supply the eye can become blocked by sickle cells.

Fever – This can be due to the damage done to the spleen.

Jaundice

Complications

Stroke – Sickle cells can block blood flow to the brain.

Acute Chest Syndrome – Occurs with an infection in the lungs or by sickle cells blocking blood vessels in the lungs. The patient may experience shortness of breath, chest pain, and fever.

Blindness – Eventually the blocked blood vessels in the eye can cause blindness over time.

Organ Damage – Organs that are chronically deprived of oxygen due to the sickle blood cells, over time nerves and organs in the patient including spleen, liver, and kidneys become damaged and this can become fatal.

Gallstones – Because blood cells have a shorter lifespan in sickle cell anemia (remember 20 days as compared to 120 days for healthy blood cells), the breakdown of the blood cells produces bilirubin. A high level of bilirubin can lead to gallstones.

Priapism – this can lead to impotence.

Diagnosis

Diagnosis is made by a blood test to check for hemoglobin S, which is the defective form of hemoglobin that is indicitive of sickle cell anemia. In the United States, this test is part of the routine newborn screening test. Adults and older children may also be tested.

Microscopic examination may also be done to check for sickle cell anemia.

Amniotic fluid may be tested to check for the sickle cell trait in the unborn baby.

If the child carries the sickle cell gene, a genetic counselor may be referred to speak and educate the parents.

Medication and Treatments

The only potential cure for sickle cell anemia is a bone marrow transplant. Not only does the procedure involve serious risk, but finding a donor can be difficult.

Therefore, avoiding a sickle cell crisis is the goal of treatment along with preventing complications and relieving symptoms.

Medications given to patients with sickle cell anemia include:

Antibiotics – It is possible that children as young as 2 months of age may be given PCN and continue with this medication until 5 years of age to prevent infections such as pneumonia.

Pain Relievers

Hydroxyurea – Usually taken daily and reduces the frequency or crisis and the need for blood transfusions.

Vaccinations – to prevent disease.

Blood transfusions – This measure increases the normal red blood cells in the patient, improving circulation. It is important to watch for iron build up since giving blood can increase the iron level in the patient. The patient may need treatment to lower iron levels as excess iron can also cause damage to the patients organs.

Oxygen – Assists in adding o2 to the blood cells and reduces shortness of breath.

Statins – Statins are primarily used to lower cholesterol, but they may also reduce inflammation which could assist with blood flow. Statins are considered experimental treatment.

Other Treatments:

Bone marrow transplant – this is also known as a stem cell transplant. The basic premise is that diseased bone marrow is replaced by healthy bone marrow.

Assessing stroke risk – transcraial ultrasound can be utilized for children as young as 2 years of age that have a high risk of stroke.

Treating complications as they arise.

Experimental Treatments:

Gene therapy – this experiment involves inserting a normal gene into the bone marrow of a patient with sickle cell anemia.

Fetal Hemoglobin production medications – Different medications are tested to see if they can boost the production of fetal hemoglobin.

Nitric Oxide – This may help in the prevention of sickle cells sticking together.

Here is a podcast on Sickle cell Anemia

Nursing Considerations

Drink water to avoid dehydration, which can lead to sickle cell crisis.

Encourage a healthy diet and folic acid supplements.

Avoid extreme hot or cold temperatures.

Regular exercise

Fly on planes only with pressurized cabins.

Use caution when traveling to a high altitude area. Supplemental oxygen may be required because there is less oxygen in higher altitude areas.

Ask healthcare provider before using over the counter medications – some OTC medications can contain ingredients such as pseudoephedrine which can constrict blood vessels.

Within 4-8 hours of nursing interventions, the client will be able to verbalize the causative factors and appropriate interventions.

Interventions

Rationals

Interventions

Rationals

Monitor pulse oximetry and report O2 saturation <92%.

These signs of symptoms of respiratory distress necessitate prompt interventions.

Assess respiratory rate. Monitor if there is an increase in anxiety, shortness of breath, cyanosis. Report any on-toward signs and symptoms immediately.

An O2 saturation of less than 92% may detect hypoxia and signals the need for supplemental oxygen.

Encourage deep breathing exercises and administer oxygen if indicated

Increases oxygen delivery to the body by mobilizing secretions and improving ventilation.

Other possible nursing diagnosis:

Risk for ineffective tissue perfusion

Deficient knowledge

Disclaimer:

Please follow your facilities guidelines and policies and procedures. The medical information on this site is provided as an information resource only, and is not to be used or relied on for any diagnostic or treatment purposes. This information is not intended to be nursing education and should not be used as a substitute for professional diagnosis and treatment.

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Anna C. RN-BC, BSN, PHN, CMSRN
Anna began writing extra materials to help her BSN and LVN students with their studies. She takes the topics that the students are learning and expands on them to try to help with their understanding of the nursing process. She is a clinical instructor for LVN and BSN students along with a critical care transport nurse.